Oxygen therapy found to ameliorate abnormal movements in a rare childhood disease (Dystonia)

Medical Xpress / Paris Brain Institute / Movement Disorders ^ | Feb. 20, 2023 | Marie Simon / Quentin Welniarz et al

[ConservativeMind]

Alternating hemiplegia of childhood is a severe neurodevelopmental disorder associated with a mutation in the ATP1A3 gene. It begins in infancy, causing symptoms that significantly impair quality of life. Young patients are affected by episodes of paralysis which can last from a few minutes to several days and involve one or alternating sides of the body. They also experience repetitive muscle contractions called dystonia, which sometimes last for hours and are often painful.

"We don't know how to help these patients. Therefore, we remain particularly attentive to the sometimes-unexpected factors that could relieve them," explains Emmanuel Flamand-Roze.

"Recently, a 25-year-old man told us after receiving an oxygen therapy device that dystonic attacks stopped 15 minutes after the oxygen was administered," says the researcher. This is a significant benefit, as this young man could experience up to 8 episodes a day, some of which could last several hours. His quality of life was improved. Oxygen therapy is a safe, highly accessible treatment that can significantly reduce the use of potent sedatives, such as midazolam, which the patient was taking at a rate of 40 doses per month.

This wave consists of a rapid phase of neuronal hyperactivity and a slower phase of electrical silence in the cortex cells. It's also associated with hypoxia—i.e., reduced oxygen availability. Rapid administration of oxygen at the time of attacks could therefore interrupt or mitigate this phenomenon.

To ensure that the association between oxygen delivery and mitigation of dystonia attacks is not coincidental, researchers must ensure that the same effect occurs in other patients under comparable conditions. Similarly, it remains to be clarified how to optimize the use of oxygen therapy, especially at home, so that it is as non-restrictive as possible.

[ConservativeMind]

Oxygen administration can help some patients with dystonia minimize their problems, reducing serious medicine use.

[ConservativeMind]

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[luckystarmom]

My daughter has dystonia. She can’t write and had to give up playing the flute. When she’s really stressed or sick she has problems in her face and legs.

She’s not as bad as others with it.